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Marfan syndrome ectopia lentis lens extraction

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Title: Marfan syndrome ectopia lentis lens extraction
Author (s): Russell Swan, MD; Alan Crandall, MD
Date: 01/01/2016
Keywords/Main Subjects: Cataract, Ectopia lentis; Marfan; Phacoemulsification; CTR; CTS
Diagnosis: Marfan’s syndrome; Ectopia lentis; Cataract
Description: This case is from a 5 year old girl who presented to ophthalmology clinic with decreased vision in both eyes, known history of Marfan’s syndrome who was found to have bilateral subluxated natural lenses. After a long discussion with the family they elected to proceed with lens extraction and intraocular lens placement.

This video demonstrates the difficulty associated with these cases due to the diffuse zonulopathy often found. While a femto-assisted capsulorhexis would have helped in this case the patient’s age and need for general anesthesia prevented the use of this technology at our institution. Given the age of the patient and the need for general anesthesia the decision was made to perform bilateral same day surgery.

A capsular tension ring and Ahmed Capsular tension segment were utilized in this case to secure the capsular bag and provide support for the lens placement. Given the likely myopic shift over time in this patient +2.0 diopters of hyperopia was targeted. Sulcus fixation was selected given that this would make potential IOL exchange easier in the future if the patient developed significant myopic shift. A 3-piece lens was thus selected as to reduce IOL related complications from the sulcus placement.

It should be noted that there are many surgical approaches to young patients with ectopia lentis related to Marfan syndrome. These include but are not limited to lensectomy with or without anterior vitrectomy, pars plana vitrectomy and lensectomy. The patient can be left aphakic, or corrected with an iris fixated IOL, anterior chamber IOL, or scleral fixated posterior chamber IOL.

Format: video
References:
Crema, A. et al. Femtosecond Laser-assisted Cataract Surgery in Patents With marfan Syndrome and Subluxated Lens. Journal Refractive Surgery. 2015 May: 31(5): 388-41
Miraldi, U., Coussa, R., Traboulsi, E. Surgical management of lens subluxation in Marfan syndrome. Journal AAPOS. 2014 Apr: 18(2):140-6
Identifier: Moran_CORE_20240
Faculty Approval by: Alan Crandall, MD

Russell Swan, MD, ©2015. For further information regarding the rights to this collection, please visit: http://morancore.utah.edu/terms-of-use/